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cardio.idiopathic-pulmonary-arterial-hypertension.v1

Idiopathic pulmonary arterial hypertension (Group 1 PAH)

cardiologychronicadultoutpatienttransition
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Group 1 idiopathic / heritable / drug-induced / CTD / HIV / portopulm PAH dossier — diagnosis by RHC; mandatory V/Q scan to exclude CTEPH. Risk-stratified therapy (4-strata ESC/ERS): low-risk → ERA + PDE5i; intermediate → triple incl. SC/inhaled prostacyclin; high-risk → IV prostacyclin + transplant referral; sotatercept (STELLAR/ZENITH) for intermediate-high/high. High-dose CCB ONLY in vasoreactivity-positive IPAH/HPAH/drug-induced (~10% of patients). Pregnancy contraindicated (~30% maternal mortality); ERAs are teratogenic; monthly pregnancy test on ERA. Next steps: (1) author manifest at prisma/seed/manifests/cardio.idiopathic-pulmonary-arterial-hypertension.v1.ts; (2) atoms; (3) design brief; (4) RxCUI verification via npm run research:rxnav:validate; (5) engine-specific test file. Calculator gaps: REVEAL Lite 2.0, ESC/ERS 4-strata risk score, COMPERA 2.0 not yet in clinical-tools-registry.ts — add before promoting to AUTHORED.

Entry points (6)

  • symptom
    Progressive exertional dyspnea unexplained by left HD or lung disease (ESC/ERS 2022)
    progressive_dyspnea_unexplained
  • symptom
    Exertional syncope / presyncope — high-risk PAH presentation (ESC/ERS 2022)
    syncope_or_presyncope_with_exertion
  • symptom
    Right HF signs — JVD, peripheral edema, ascites, hepatic congestion (ESC/ERS 2022)
    right_sided_HF_signs
  • imaging
    Echo with RVSP >=40 mmHg, dilated RV, septal flattening, TAPSE <=17 mm (ESC/ERS 2022 Humbert)
    echo_elevated_RVSP_or_RV_dysfunction
  • imaging
    RHC: mPAP ≥20 + PVR >2 WU + PCWP ≤15
    rhc_confirmed_pah
  • problem_list
    Known Group 1 PAH — risk reassessment / titration
    group1_pah_existing

Required inputs (23)

  • agerequired
    demographic • used at CONTEXT
    Age + sex shapes risk score; women 4:1 in IPAH (ESC/ERS 2022)
  • sbprequired
    vital • used at CONTEXT
    Systemic SBP <100 + tachycardia = REVEAL high-risk (Benza CHEST 2012)
  • hrrequired
    vital • used at CONTEXT
    HR >=90 = high-risk (REVEAL Lite 2.0)
  • spo2required
    vital • used at CONTEXT
    Resting SpO2 + 6MWT desaturation; supplemental O2 if <90% (ESC/ERS 2022)
  • six_min_walk_distancerequired
    vital • used at RISK_STRATIFICATION
    6MWD <165 m = high-risk; >440 m = low-risk per 4-strata score
  • nt_probnprequired
    lab • used at INITIAL_WORKUP
    NT-proBNP <300 = low-risk; >1100 = high-risk; tracks RV strain (ESC/ERS 2022 4-strata)
  • creatininerequired
    lab • used at CONTEXT
    eGFR for diuretic + drug clearance (ESC/ERS 2022)
  • bnp_or_nt_probnp_trend
    lab • used at MONITORING
    Serial BNP for monitoring response (ESC/ERS 2022)
  • lftrequired
    lab • used at CONTEXT
    ERA hepatotoxicity baseline + monthly monitoring (bosentan REMS; macitentan less) per ESC/ERS 2022
  • pregnancy_testrequired
    lab • used at CONTEXT
    PAH + pregnancy = ~30% maternal mortality; ERAs teratogenic; mandatory monthly during ERA (ESC/ERS 2022)
  • autoimmune_panel
    lab • used at BRANCHING_WORKUP
    CTD-PAH workup — ANA, anti-Scl70, anti-centromere, anti-RNP for SSc/MCTD; APS (ESC/ERS 2022)
  • hiv_test
    lab • used at BRANCHING_WORKUP
    HIV-PAH is Group 1 subtype (ESC/ERS 2022)
  • hepatitis_panel
    lab • used at BRANCHING_WORKUP
    Portal HTN + Hep B/C -> portopulmonary HTN (Group 1) (ESC/ERS 2022)
  • echorequired
    imaging • used at INITIAL_WORKUP
    Initial screening; RVSP, TAPSE, RV/LV ratio, septal flattening, pericardial effusion (ESC/ERS 2022)
  • rhcrequired
    imaging • used at DIFFERENTIAL
    GOLD STANDARD diagnosis: mPAP >=20, PVR >2 WU, PCWP <=15; vasoreactivity test in IPAH only (ESC/ERS 2022 Humbert)
  • vq_scanrequired
    imaging • used at BRANCHING_WORKUP
    Mandatory to exclude CTEPH (Group 4) before labelling Group 1 (ESC/ERS 2022 Class I)
  • pftrequired
    imaging • used at BRANCHING_WORKUP
    Exclude Group 3 (lung disease) (ESC/ERS 2022)
  • ecg
    imaging • used at INITIAL_WORKUP
    RVH, RAD, RBBB, RV strain — supportive (ESC/ERS 2022)
  • cardiac_MRI
    imaging • used at INITIAL_WORKUP
    RV mass, function, LGE — prognostic adjunct (ESC/ERS 2022)
  • who_functional_classrequired
    history • used at RISK_STRATIFICATION
    WHO FC I-IV — primary driver of treatment intensity + risk (ESC/ERS 2022 4-strata)
  • methamphetamine_or_anorexigen_use
    history • used at CONTEXT
    Drug- and toxin-induced PAH (Group 1.3) (ESC/ERS 2022)
  • family_history_pah_bmpr2
    history • used at CONTEXT
    Heritable PAH (BMPR2/ACVRL1/ENG/SMAD9/CAV1/KCNK3) (ESC/ERS 2022)
  • current_medsrequired
    medication • used at CONTEXT
    Detect existing PAH therapy + nitrates / PDE5 contraindications + ERA-induced LFT changes (ESC/ERS 2022)

12-phase flow (12)

  1. 1FRAME
    Confirm Group 1 PAH per 2022 ESC/ERS — mPAP ≥20 + PVR >2 WU + PCWP ≤15 + exclusion of Groups 2–5
    inputs: rhc
    advance: Group 1 confirmed; CTEPH and Groups 2/3/5 excluded
  2. 2ENTRY
    Recognise screening trigger — unexplained dyspnea, exertional syncope, RV dilation on echo
    inputs: age
    advance: screening trigger captured
  3. 3CONTEXT
    Functional class, comorbidities (CTD, HIV, portal HTN, drug exposure, family history); medications; pregnancy status
    inputs: sbp, hr, spo2, creatinine, lft, pregnancy_test, methamphetamine_or_anorexigen_use, family_history_pah_bmpr2, current_meds
    advance: context complete
  4. 4RED_FLAGS
    High-risk presentation: WHO FC IV, syncope, RV failure (JVD, ascites, hypotension), pre-syncope on exertion, lactate elevation; pregnancy
    inputs: sbp, hr, who_functional_class
    actions: phtn_acute, cardiogenic_shock
    advance: red flags addressed or routed
  5. 5INITIAL_WORKUP
    Echo, NT-proBNP, BMP, LFT, PFT, V/Q scan (mandatory CTEPH exclusion), CT chest, ECG, autoimmune panel, HIV, hepatitis
    inputs: echo, nt_probnp, pft, vq_scan, autoimmune_panel, hiv_test, hepatitis_panel
    actions: panel.cardiac, panel.renal, panel.lft
    advance: initial workup complete
  6. 6BRANCHING_WORKUP
    RHC with vasoreactivity testing (in IPAH/HPAH/drug-induced only — not CTD/HIV/portopulm); cardiac MRI; sleep study; genetic testing if HPAH suspected
    inputs: rhc
    actions: phtn_acute
    advance: RHC complete + vasoreactivity tested + Groups 2/3/4/5 excluded
  7. 7DIFFERENTIAL
    Confirm Group 1 subtype per ESC/ERS 2022: 1.1 idiopathic, 1.2 heritable, 1.3 drug/toxin, 1.4 CTD/HIV/portopulm/CHD/schistosomiasis, 1.5 PVOD/PCH
    inputs: rhc
    advance: subtype assigned
  8. 8RISK_STRATIFICATION
    ESC/ERS 4-strata risk score (low / intermediate-low / intermediate-high / high) using WHO FC, 6MWD, NT-proBNP, RAP, CI, SvO2, RV imaging; REVEAL Lite 2.0
    inputs: who_functional_class, six_min_walk_distance, nt_probnp
    advance: risk stratum assigned
  9. 9TREATMENT
    Risk-based therapy per ESC/ERS 2022: low-risk → ERA + PDE5i (AMBITION Sitbon NEJM 2015); intermediate-risk → triple with SC/inhaled prostacyclin; high-risk → IV prostacyclin + triple; sotatercept (STELLAR NEJM 2023 / ZENITH) for intermediate-high/high; lung transplant referral
    inputs: who_functional_class, six_min_walk_distance, nt_probnp, sbp, lft
    advance: risk-matched regimen prescribed; supportive (diuretic, O2, anticoag) decided
  10. 10DISPOSITION
    PAH expert centre referral mandatory per ESC/ERS 2022; transplant evaluation if intermediate-high/high after triple therapy + sotatercept
    inputs: who_functional_class
    advance: specialty + transplant referrals made if applicable
  11. 11MONITORING
    q3 mo follow-up with WHO FC + 6MWD + NT-proBNP + echo; q6 mo RHC if intermediate/high or after escalation; LFT monthly on bosentan; pregnancy test monthly on ERA
    inputs: who_functional_class, six_min_walk_distance, nt_probnp, lft
    actions: panel.lft
    advance: monitoring schedule documented
  12. 12FOLLOWUP
    PAH-specific exercise rehab; pneumococcal/influenza/COVID vaccinations; contraception counselling; mental-health support
    advance: follow-up scheduled with PAH expert center